Choledochal Cyst Disease in Children and Adults: A 30-Year Single-Institution Experience

Barish H. Edil, John L. Cameron, Sushanth Reddy, Yingwei Lum, Pamela A. Lipsett, Hari Nathan, Timothy M. Pawlik, Michael A. Choti, Christopher L. Wolfgang, Richard D. Schulick

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Abstract

Background: The reported incidence of choledochal cyst (CC) disease varies greatly based on geography. Most large series on CC originate from East Asia. So our understanding of the presentation and natural history of CC disease in western societies is less well characterized. Recognition and surgical treatment are important because of the significant longterm risks of developing cholangiocarcinoma. We report here the largest single-institution western experience with CC disease. Study Design: Ninety-two patients with CC disease who were seen at our institution between 1976 and 2006 were included in this study. Potential differences between children and adults (defined as 16 years old or older) were specifically evaluated. Results: There were 19 children and 73 adults; 90% were girls/women with type I cysts. Adults were more likely to present with abdominal pain (97% versus 63%, p < 0.001), and children were more likely to present with jaundice (71% versus 25%, p = 0.001). Surgical management most commonly involved cyst excision and Roux-en-Y hepaticojejunostomy reconstruction. Four adults (three with cholangiocarcinoma and one with gall bladder cancer) and one child (with embryonal rhabdomyosarcoma) had malignancies associated with their CC at the time of excision. Two patients (with type IV and type V disease) developed cholangiocarcinoma after surgical management. Another patient died of pancreatic adenocarcinoma 21 years after excision of her type I cyst. There was no operative or hospital mortality, and no patient who underwent complete cyst excision developed cholangiocarcinoma during a mean followup of 10 years. Conclusions: Western CC disease has a similar demographic profile as that seen in Asia. CCs are more frequently found in adults and girls/women and are associated with a longterm risk of developing cholangiocarcinoma. Presentation differs between adults and children. After complete cyst excision, no patients developed cholangiocarcinoma.

Original languageEnglish (US)
Pages (from-to)1000-1005
Number of pages6
JournalJournal of the American College of Surgeons
Volume206
Issue number5
DOIs
StatePublished - May 2008

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Choledochal Cyst
Cholangiocarcinoma
Cysts
Embryonal Rhabdomyosarcoma
Gallbladder Neoplasms
Geography
Far East
Hospital Mortality
Jaundice
Abdominal Pain
Adenocarcinoma
Demography
Incidence

ASJC Scopus subject areas

  • Surgery

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Edil, B. H., Cameron, J. L., Reddy, S., Lum, Y., Lipsett, P. A., Nathan, H., ... Schulick, R. D. (2008). Choledochal Cyst Disease in Children and Adults: A 30-Year Single-Institution Experience. Journal of the American College of Surgeons, 206(5), 1000-1005. https://doi.org/10.1016/j.jamcollsurg.2007.12.045

Choledochal Cyst Disease in Children and Adults : A 30-Year Single-Institution Experience. / Edil, Barish H.; Cameron, John L.; Reddy, Sushanth; Lum, Yingwei; Lipsett, Pamela A.; Nathan, Hari; Pawlik, Timothy M.; Choti, Michael A.; Wolfgang, Christopher L.; Schulick, Richard D.

In: Journal of the American College of Surgeons, Vol. 206, No. 5, 05.2008, p. 1000-1005.

Research output: Contribution to journalArticle

Edil, BH, Cameron, JL, Reddy, S, Lum, Y, Lipsett, PA, Nathan, H, Pawlik, TM, Choti, MA, Wolfgang, CL & Schulick, RD 2008, 'Choledochal Cyst Disease in Children and Adults: A 30-Year Single-Institution Experience', Journal of the American College of Surgeons, vol. 206, no. 5, pp. 1000-1005. https://doi.org/10.1016/j.jamcollsurg.2007.12.045
Edil, Barish H. ; Cameron, John L. ; Reddy, Sushanth ; Lum, Yingwei ; Lipsett, Pamela A. ; Nathan, Hari ; Pawlik, Timothy M. ; Choti, Michael A. ; Wolfgang, Christopher L. ; Schulick, Richard D. / Choledochal Cyst Disease in Children and Adults : A 30-Year Single-Institution Experience. In: Journal of the American College of Surgeons. 2008 ; Vol. 206, No. 5. pp. 1000-1005.
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abstract = "Background: The reported incidence of choledochal cyst (CC) disease varies greatly based on geography. Most large series on CC originate from East Asia. So our understanding of the presentation and natural history of CC disease in western societies is less well characterized. Recognition and surgical treatment are important because of the significant longterm risks of developing cholangiocarcinoma. We report here the largest single-institution western experience with CC disease. Study Design: Ninety-two patients with CC disease who were seen at our institution between 1976 and 2006 were included in this study. Potential differences between children and adults (defined as 16 years old or older) were specifically evaluated. Results: There were 19 children and 73 adults; 90{\%} were girls/women with type I cysts. Adults were more likely to present with abdominal pain (97{\%} versus 63{\%}, p < 0.001), and children were more likely to present with jaundice (71{\%} versus 25{\%}, p = 0.001). Surgical management most commonly involved cyst excision and Roux-en-Y hepaticojejunostomy reconstruction. Four adults (three with cholangiocarcinoma and one with gall bladder cancer) and one child (with embryonal rhabdomyosarcoma) had malignancies associated with their CC at the time of excision. Two patients (with type IV and type V disease) developed cholangiocarcinoma after surgical management. Another patient died of pancreatic adenocarcinoma 21 years after excision of her type I cyst. There was no operative or hospital mortality, and no patient who underwent complete cyst excision developed cholangiocarcinoma during a mean followup of 10 years. Conclusions: Western CC disease has a similar demographic profile as that seen in Asia. CCs are more frequently found in adults and girls/women and are associated with a longterm risk of developing cholangiocarcinoma. Presentation differs between adults and children. After complete cyst excision, no patients developed cholangiocarcinoma.",
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